Prevalence and prognostic significance of left ventricular reverse remodeling in dilated cardiomyopathy receiving tailored medical treatment.

The purpose of this study was to determine the prevalence and prognostic role of left ventricular reverse remodeling (LVRR) in idiopathic dilated cardiomyopathy (IDCM).Tailored medical therapy can lead to LVRR in IDCM. The prevalence and prognostic impact of LVRR remain unclear.We consecutively enrolled 361 IDCM patients. LVRR was defined as a left ventricular ejection fraction increase of 6510 U or a left ventricular ejection fraction of 6550\% and a decrease in indexed left ventricular end-diastolic diameter of 6510\% or indexed left ventricular end-diastolic diameter of 6533 mm/m(2) at 24 months (range 9 to 36 months). Follow-up echocardiographic data were available for 242 patients (67\%), 34 (9\%) died/underwent heart transplantation (HTx) before re-evaluation, and 85 (24\%) did not have a complete re-evaluation. After re-evaluation, the surviving patients were followed for 110 \ub1 53 months; there were 55 deaths (23\%) and 32 HTx (13\%).LVRR was found in 89 of 242 patients (37\%). Baseline predictors of LVRR were higher systolic blood pressure (p = 0.047) and the absence of left bundle branch block (p = 0.009). When added to a prognostic baseline model including male sex, heart failure duration, New York Heart Association functional classes III to IV, LVEF, significant mitral regurgitation, and beta-blockers, LVRR, New York Heart Association functional classes III to IV, and significant mitral regurgitation after 24 months emerged as independent predictors of death/HTx and heart failure death/HTx. The model including follow-up variables showed additional prognostic power with respect to baseline model (for death/HTx, area under the curve: 0.80 vs. 0.70, respectively, p = 0.004). Furthermore, only LVRR was significantly associated with sudden death/major ventricular arrhythmia in the long-term.LVRR characterized approximately one-third of IDCM patients surviving 2 years while receiving optimal medical therapy and allowed a more accurate long-term prognostic stratification of the disease

Cardiomiopatia dilatativa: una, nessuna, centomila

Cardiomiopatia dilatativ

Echocardiographic evaluation of systolic and mean pulmonary artery pressure in the follow-up of patients with pulmonary hypertension.

To identify a correction of the modified Bernoulli formula used to estimate systolic and mean pulmonary artery pressure [sPAP and mPAP; respectively: sPAP = 4 7 TRv (tricuspid regurgitation velocity)(2)+ RAP (right atrial pressure); and mPAP = 0.61sPAP + 2], applicable in the follow-up of pulmonary hypertension (PH) patients.From January 1979 to December 2009, 60 patients with precapillary (class I and IV) and 'out of proportion' PH were consecutively enrolled in the PH Registry of Trieste. All patients underwent both echocardiographic and right heart catheter evaluation. We used a simple-linear-regression method in order to compare sPAP and mPAP Doppler-estimated values with the respective right-heart catheterization invasive variables. The comparison of the estimated with the traditional modified Bernoulli formula echo-Doppler data and the effective invasive values confirmed a significant association between them (for sPAP P< 0.001; for mPAP P= 0.006). Simple-linear-regression-derived formulas were sPAP = 1.07 7 (4TRv(2)+ RAP) + 7.4 (1) and mPAP = 1.1 7 (0.61sPAP + 2) + 2.5 (2). These regression-corrected formulas were validated in an external population of PH patients.Our data suggest that formulas (1) and (2) could be more reliable with respect to the traditional modified Bernoulli equation, when estimating echocardiographically sPAP and mPAP in patients with PH confirmed by right-heart catheterization

Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry.

We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete.A cohort of 96 ARVC patients (68\% males, 35 \ub1 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33\%) and 24\% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50\%). During a mean follow-up of 128 \ub1 92 months, 20 patients (21\%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95\% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95\% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95\% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95\% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04).In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone